5 results on '"Chiu, W.Z. (Wang Zheng)"'
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2. Midcingulate involvement in progressive supranuclear palsy and tau positive frontotemporal dementia
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Chiu, W.Z. (Wang Zheng), Papma, J.M. (Janne), Koning, I. (Inge) de, Donker Kaat, L. (Laura), Seelaar, H. (Harro), Reijs, A.E.M. (Ambroos), Valkema, R. (Roelf), Hasan, D. (Djo), Boon, A.J.W. (Andrea), Swieten, J.C. (John) van, Chiu, W.Z. (Wang Zheng), Papma, J.M. (Janne), Koning, I. (Inge) de, Donker Kaat, L. (Laura), Seelaar, H. (Harro), Reijs, A.E.M. (Ambroos), Valkema, R. (Roelf), Hasan, D. (Djo), Boon, A.J.W. (Andrea), and Swieten, J.C. (John) van
- Abstract
Background Progressive supranuclear palsy (PSP) patients often exhibit cognitive decline and behavioural changes during the disease course. In a subset, these symptoms may be the presenting manifestation and can be similar to those in frontotemporal dementia (FTD). However, correlation studies between quantitative imaging measures and detailed neuropsychological assessment are scarce. The aim of this study was to investigate the functional role of affected brain regions in cognition in PSP compared with controls and subsequently examine these regions in FTD patients with known tau pathology (FTD tau). Methods 21 PSP patients, 27 healthy controls and 11 FTD tau patients were enrolled. All participants underwent neuropsychological testing and technetium99m-hexamethyl-propylenamine-oxime single photon emission CT. Regression slope analyses were performed in statistical parametric mapping to find significant associations between neuropsychological test results and brain perfusion. Results PSP patients showed hypoperfusion in the midcingulate cortex (MCC) of which the posterior part correlated with Stroop III and Weigl. In FTD tau patients, MCC involvement was located more anterior and correlated with Stroop III and Wisconsin Card Sorting Test concepts. The degree of hypoperfusion in the anterior cortex and MCC in the disorders differed in the subgenual anterior cingulate cortex only. Conclusions Th
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- 2012
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3. Identification of common variants influencing risk of the tauopathy progressive supranuclear palsy
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Hoglinger, G. (Gunter), Melhem, N.M. (Nadine), Dickson, D. (Dennis), Sleiman, P.M.A. (Patrick), Wang, L.S. (Li-San), Klei, L. (Lambertus), Rademakers, R. (Rosa), Silva, R. (Rohan) de, Litvan, I. (Irene), Riley, D.E. (David), Swieten, J.C. (John) van, Heutink, P. (Peter), Wszolek, Z.K. (Zbigniew), Uitti, R.J. (Ryan), Vandrovcova, J. (Jana), Hurtig, H.I. (Howard), Gross, R.G. (Rachel), Maetzler, W. (Walter), Goldwurm, S. (Stefano), Tolosa, E., Borroni, B. (Barbara), Pastor, P. (Pau), Cantwell, L.B. (Laura), Han, M.R., Dillman, A. (Allissa), Brug, M.P. (Marcel) van der, Gibbs, J. (Raphael), Cookson, M.R. (Mark), Hernandez, D.G. (Dena), Singleton, A. (Andrew), Farrer, M.J. (Matthew), Yu, C.-E. (Changen), Golbe, L.I. (Lawrence), Revesz, T. (Tamas), Hardy, J. (John), Lees, A.J. (Andrew), Devlin, B. (Bernie), Hakonarson, H. (Hakon), Müller, U. (Ulrich), Schellenberg, G.D. (Gerard), Albin, R.L. (Roger), Alonso, E. (Elena), Apfelbacher, M. (Manuela), Arnold, S.E. (Steven), Avila, J. (Jesús), Beach, T.G. (Thomas), Beecher, S. (Sherry), Berg, D. (Daniela), Bird, T.D. (Thomas), Bogdanović, N. (Nenad), Boon, A.J.W. (Andrea), Bordelon, Y. (Yvette), Brice, A. (Alexis), Budka, H. (Herbert), Canesi, M. (Margherita), Chiu, W.Z. (Wang Zheng), Cilia, R. (Roberto), Colosimo, C. (Carlo), Deyn, P.P. (Peter) de, Yebenes, J.G. de, Donker Kaat, L. (Laura), Duara, R. (Ranjan), Durr, A., Engelborghs, S. (Sebastiaan), Fabbrini, G. (Giovanni), Finch, N.A. (Nicole), Flook, R. (Robyn), Frosch, M.P. (Matthew), Gaig, C., Galasko, D. (Douglas), Gasser, T. (Thomas), Gearing, M. (Marla), Geller, E.T. (Evan), Ghetti, B. (Bernardino), Graff-Radford, N.R. (Neill), Grossman, M. (Murray), Hall, D.A. (Deborah), Hazrati, L.-N., Höllerhage, M. (Matthias), Jankovic, J. (Joseph), Juncos, J.L. (Jorge), Karydas, A. (Anna), Kretzschmar, H.A. (Hans), Leber, I. (Isabelle), Lee, V.M.Y. (Virginia), Lieberman, A.P. (Andrew), Lyons, K.E. (Kelly), Mariani, C. (Claudio), Masliah, E. (Eliezer), Massey, L.A. (Luke), McLean, C.A. (Catriona), Meucci, N. (Nicoletta), Miller, B.L. (Bruce), Mollenhauer, B. (Brit), Möller, J.C. (Jens), Morris, H. (Huw), O'Sullivan, S.S. (Sean), Oertel, W., Ottaviani, D. (Donatella), Padovani, A. (Alessandro), Pahwa, R. (Rajesh), Pezzoli, G. (Gianni), Pickering-Brown, S. (Stuart), Poewe, W. (Werner), Rabano, A. (Alberto), Rajput, A. (Alex), Reich, S.G. (Stephen), Respondek, G. (Gesine), Roeber, S. (Sigrun), Rohrer, J.D. (Jonathan), Ross, O.A. (Owen), Rossor, M. (Martin), Sacilotto, G. (Giorgio), Seeley, W.W. (William), Seppi, K. (Klaus), Silveira-Moriyama, L. (Laura), Spina, S. (Salvatore), Srulijes, K. (Karin), St. George-Hyslop, P. (Peter), Stamelou, M. (Maria), Standaert, D.G. (David), Tesei, S. (Silvana), Tourtellotte, W.W. (Wallace), Trenkwalder, C. (Claudia), Troakes, C. (Claire), Trojanowski, J.Q. (John), Troncoso, J.C. (Juan), Deerlin, V.M. (Vivianna), Vonsattel, J.P.G., Wenning, G.K. (Gregor), White III, C.L. (Charles), Winter, P. (Pia), Zarow, C. (Chris), Zecchinelli, A.L. (Anna), Antonini, A. (Angelo), Hoglinger, G. (Gunter), Melhem, N.M. (Nadine), Dickson, D. (Dennis), Sleiman, P.M.A. (Patrick), Wang, L.S. (Li-San), Klei, L. (Lambertus), Rademakers, R. (Rosa), Silva, R. (Rohan) de, Litvan, I. (Irene), Riley, D.E. (David), Swieten, J.C. (John) van, Heutink, P. (Peter), Wszolek, Z.K. (Zbigniew), Uitti, R.J. (Ryan), Vandrovcova, J. (Jana), Hurtig, H.I. (Howard), Gross, R.G. (Rachel), Maetzler, W. (Walter), Goldwurm, S. (Stefano), Tolosa, E., Borroni, B. (Barbara), Pastor, P. (Pau), Cantwell, L.B. (Laura), Han, M.R., Dillman, A. (Allissa), Brug, M.P. (Marcel) van der, Gibbs, J. (Raphael), Cookson, M.R. (Mark), Hernandez, D.G. (Dena), Singleton, A. (Andrew), Farrer, M.J. (Matthew), Yu, C.-E. (Changen), Golbe, L.I. (Lawrence), Revesz, T. (Tamas), Hardy, J. (John), Lees, A.J. (Andrew), Devlin, B. (Bernie), Hakonarson, H. (Hakon), Müller, U. (Ulrich), Schellenberg, G.D. (Gerard), Albin, R.L. (Roger), Alonso, E. (Elena), Apfelbacher, M. (Manuela), Arnold, S.E. (Steven), Avila, J. (Jesús), Beach, T.G. (Thomas), Beecher, S. (Sherry), Berg, D. (Daniela), Bird, T.D. (Thomas), Bogdanović, N. (Nenad), Boon, A.J.W. (Andrea), Bordelon, Y. (Yvette), Brice, A. (Alexis), Budka, H. (Herbert), Canesi, M. (Margherita), Chiu, W.Z. (Wang Zheng), Cilia, R. (Roberto), Colosimo, C. (Carlo), Deyn, P.P. (Peter) de, Yebenes, J.G. de, Donker Kaat, L. (Laura), Duara, R. (Ranjan), Durr, A., Engelborghs, S. (Sebastiaan), Fabbrini, G. (Giovanni), Finch, N.A. (Nicole), Flook, R. (Robyn), Frosch, M.P. (Matthew), Gaig, C., Galasko, D. (Douglas), Gasser, T. (Thomas), Gearing, M. (Marla), Geller, E.T. (Evan), Ghetti, B. (Bernardino), Graff-Radford, N.R. (Neill), Grossman, M. (Murray), Hall, D.A. (Deborah), Hazrati, L.-N., Höllerhage, M. (Matthias), Jankovic, J. (Joseph), Juncos, J.L. (Jorge), Karydas, A. (Anna), Kretzschmar, H.A. (Hans), Leber, I. (Isabelle), Lee, V.M.Y. (Virginia), Lieberman, A.P. (Andrew), Lyons, K.E. (Kelly), Mariani, C. (Claudio), Masliah, E. (Eliezer), Massey, L.A. (Luke), McLean, C.A. (Catriona), Meucci, N. (Nicoletta), Miller, B.L. (Bruce), Mollenhauer, B. (Brit), Möller, J.C. (Jens), Morris, H. (Huw), O'Sullivan, S.S. (Sean), Oertel, W., Ottaviani, D. (Donatella), Padovani, A. (Alessandro), Pahwa, R. (Rajesh), Pezzoli, G. (Gianni), Pickering-Brown, S. (Stuart), Poewe, W. (Werner), Rabano, A. (Alberto), Rajput, A. (Alex), Reich, S.G. (Stephen), Respondek, G. (Gesine), Roeber, S. (Sigrun), Rohrer, J.D. (Jonathan), Ross, O.A. (Owen), Rossor, M. (Martin), Sacilotto, G. (Giorgio), Seeley, W.W. (William), Seppi, K. (Klaus), Silveira-Moriyama, L. (Laura), Spina, S. (Salvatore), Srulijes, K. (Karin), St. George-Hyslop, P. (Peter), Stamelou, M. (Maria), Standaert, D.G. (David), Tesei, S. (Silvana), Tourtellotte, W.W. (Wallace), Trenkwalder, C. (Claudia), Troakes, C. (Claire), Trojanowski, J.Q. (John), Troncoso, J.C. (Juan), Deerlin, V.M. (Vivianna), Vonsattel, J.P.G., Wenning, G.K. (Gregor), White III, C.L. (Charles), Winter, P. (Pia), Zarow, C. (Chris), Zecchinelli, A.L. (Anna), and Antonini, A. (Angelo)
- Abstract
Progressive supranuclear palsy (PSP) is a movement disorder with prominent tau neuropathology. Brain diseases with abnormal tau deposits are called tauopath
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- 2011
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4. Symmetrical corticobasal syndrome caused by a novel c.314dup progranulin mutation
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Dopper, E.G.P. (Elise), Seelaar, H. (Harro), Chiu, W.Z. (Wang Zheng), Koning, I. (Inge) de, Minkelen, R. (Rick) van, Baker, M.C. (Matthew), Rozemuller, A.J.M. (Annemieke), Rademakers, R. (Rosa), Swieten, J.C. (John) van, Dopper, E.G.P. (Elise), Seelaar, H. (Harro), Chiu, W.Z. (Wang Zheng), Koning, I. (Inge) de, Minkelen, R. (Rick) van, Baker, M.C. (Matthew), Rozemuller, A.J.M. (Annemieke), Rademakers, R. (Rosa), and Swieten, J.C. (John) van
- Abstract
Corticobasal syndrome (CBS) is characterised by asymmetrical parkinsonism and cognitive impairment. The underlying pathology varies between corticobasal degeneration, progressive supranuclear palsy, Alzheimer's disease, Creutzfeldt-Jakob disease and frontotemporal lobar degeneration sometimes in association with GRN mutations. A 61-year-old male underwent neurological examination, neuropsychological assessment, MRI, and HMPAO-SPECT at our medical centre. After his death at the age of 63, brain autopsy, genetic screening and mRNA expression analysis were performed. The patient presented with slow progressive walking disabilities, non-fluent language problems, behavioural changes and forgetfulness. His family history was negative. He had primitive reflexes, rigidity of his arms and postural instability. Later in the disease course he developed dystonia of his left leg, pathological crying, mutism and dysphagia. Neuropsychological assessment revealed prominent ideomotor and ideational apraxia, executive dysfunction, non-fluent aphasia and memory deficits. Neuroimaging showed symmetrical predominant frontoparietal atrophy and hypoperfusion. Frontotemporal lobar degeneration (FTLD)-TDP type 3 pathology was found at autopsy. GRN sequencing revealed a novel frameshift mutation c.314dup, p.Cys105fs and GRN mRNA levels showed a 50% decrease. We found a novel GRN mutation in a patient with an atypical (CBS) presentation with symmetric neuroimaging findings. GRN mutations are an important cause of CBS associated with FTLD-TDP type 3 pathology, sometimes in sporadic cases. Screening for GRN mutations should also be considered in CBS patients without a positive family history.
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- 2011
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5. Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration
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Seelaar, H. (Harro), Klijnsma, K.Y. (Kirsten), Koning, I. (Inge) de, Lugt, A. (Aad) van der, Chiu, W.Z. (Wang Zheng), Azmani, A. (Asma), Rozemuller, A.J.M. (Annemieke), Swieten, J.C. (John) van, Seelaar, H. (Harro), Klijnsma, K.Y. (Kirsten), Koning, I. (Inge) de, Lugt, A. (Aad) van der, Chiu, W.Z. (Wang Zheng), Azmani, A. (Asma), Rozemuller, A.J.M. (Annemieke), and Swieten, J.C. (John) van
- Abstract
Frontotemporal lobar degeneration (FTLD) is a clinically, genetically and pathologically heterogeneous disorder. Within FTLD with ubiquitin-positive inclusions (FTLD-U), a new pathological subtype named FTLD-FUS was recently found with fused in sarcoma (FUS) positive, TDP-43-negative inclusions, and striking atrophy of the caudate nucleus. The aim of this study was to determine the frequency of FTLD-FUS in our pathological FTLD series, and to describe the clinical, neuroimaging and neuropathological features of FTLD-FUS, especially caudate atrophy. Demographic and clinical data collected prospectively from 387 patients with frontotemporal dementia (FTD) yielded 74 brain specimens. Immunostaining was carried out using a panel of antibodies, including AT-8, ubiquitin, p62, FUS, and TDP-43. Cortical and caudate atrophy on MRI (n = 136) was rated as normal, mildmoderate or severe. Of the 37 FTLD-U cases, 33 were reclassified as FTLD-TDP and four (0.11, 95%: 0.00-0.21) as FTLD-FUS, with ubiquitin and FUS-positive, p62 and TDP-43-negative neuronal intranuclear inclusions (NII). All four FTLD-FUS cases had a negative family history, behavioural variant FTD (bvFTD), and three had an age at onset ≤40 years. MRI revealed mild-moderate or severe caudate atrophy in all, with a mean duration from onset till MRI of 63 months (range 16-119 months). In our total clinical FTD cohort, we found 11 pati
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- 2010
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